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Table 1 Mouse models for laminin α2 chain deficiency.

From: Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies

Mouse model

Mutation/protein product

Phenotype

Ref

dy/dy

Unknown spontaneous mutation/reduced expression of seemingly normal α2 chain

Lethal within 6 months of age. Moderate muscular dystrophy; peripheral neuropathy; defective central nervous system myelination; hearing loss; aberrant thymocyte development

[11, 12, 84, 121, 122]

dy2J/dy2J

Spontaneous mutation in LN domaina/slightly reduced expression of truncated α2 chain devoid of LN domain

Normal lifespan. Mild muscular dystrophy; peripheral neuropathy

[85, 86]

dyW/dyW

Knock- out/severely reduced expression of truncated α2 chain devoid of LN domain

Lethal at 10 to 15 weeks of age. Severe muscular dystrophy; peripheral neuropathy

[43, 83]

dy3K/dy3K

Knock-out/complete deficiency

Lethal at 4 weeks of age. Severe muscular dystrophy; peripheral neuropathy; impaired spermatogenesis; defective odontoblast differentiation

[42, 123–125]

dynmj417/dynmf417

N-ethyl-N-nitrosourea-induced point mutation in LN domain/normal levels

Normal lifespan. Mild muscular dystrophy; peripheral neuropathy

[87]

dyPas/dyPas(now extinct)

Spontaneous retrotransposal insertion/severe deficiency

Died at 13 weeks of age. Severe muscular dystrophy; peripheral neuropathy

[126]

  1. aLN = laminin N-terminal domain.